An assessment of Fontan procedure outcomes and how we can reduce post-Fontan complications

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Adult Congenital Heart Disease

Background:

• The Fontan procedure significantly improves survival for single ventricle physiology but presents long-term challenges.
• Patients face high rates of cardiac and extracardiac complications, impacting quality of life and longevity.
• Aging Fontan populations necessitate ongoing monitoring and comprehensive care for late complications.

Purpose of the Study:

• To review current evidence on long-term outcomes following Fontan-type surgery.
• To detail the pathophysiology of the Fontan circuit and management of common complications.
• To emphasize the importance of specialist multidisciplinary care for Fontan patients.

Main Methods:

• Literature review of established clinical practice and recent advances.
• Critical evaluation of incidence, severity, and management strategies for complications.
• Focus on evidence-based approaches and the role of multidisciplinary teams.

Main Results:

• Long-term outcomes are challenged by significant short and long-term complications.
• Common issues include arrhythmias, heart failure, liver disease, and need for reintervention/transplantation.
• Late cardiac and extracardiac complications are prevalent in the aging Fontan population.

Conclusions:

• Optimal care for Fontan patients relies on comprehensive multidisciplinary management.
• Novel therapies show potential but should complement, not replace, established care models.
• Continued research is crucial for refining guidelines and improving outcomes for individuals with Fontan circulation.